The association between the congenital heart diseases and congenital anomalies of the kidney and the urinary tract in non-syndromic children.

KeleŞ Alp E., Dönmez M. İ., Alp H., Elmacı A. M.

Congenital anomalies, 2021 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume:
  • Publication Date: 2021
  • Doi Number: 10.1111/cga.12443
  • Journal Name: Congenital anomalies
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), BIOSIS, EMBASE, MEDLINE
  • Keywords: congenital anomalies of the kidney and tract, congenital heart disease, echocardiography, nonsyndromic, routine, urinary, PRENATAL-DIAGNOSIS, PREVALENCE, BIRTH, MALFORMATIONS, DEFECTS, INFANTS, ATLANTA
  • Istanbul University Affiliated: Yes


Several congenital heart diseases (CHD) are present in children with congenital anomalies of the kidney and the urinary tract (CAKUT) in syndromic or nonsyndromic patterns. The purpose of this study was to identify nonsyndromic children with CAKUT who required echocardiographic evaluation and to find the prevalence of CHD in these patients. Retrospective chart review of nonsyndromic patients with CAKUT between 2012 and 2018 was conducted. Types of congenital anomalies of the kidney and urinary tract and echocardiogram reports of the patients, age, gender, prematurity, prenatal diagnosis of CAKUT, and family history were noted. Among 806 children with CAKUT, 135 patients underwent transthoracic echocardiographic evaluation and CHD was detected in 91 (11.2%). The most common type of CHD was secundum type atrial septal defect that was found in 73 (80.2%) patients. On the other hand, the most frequent types of congenital anomalies of the kidney and the urinary tract were isolated hydronephrosis and primary vesicoureteral reflux (61.4% and 12.8%, respectively). Our results showed that 75.8% of the patients with congenital heart diseases had isolated hydronephrosis. Finally, only 18 (2.2%) cases out of 806 patients that were evaluated via transthoracic echocardiography due to the clinical findings and family history had a CHD that required close (<6 months) follow-up. Congenital heart diseases that required close follow-up are rarely observed in nonsyndromic patients with CAKUT. Therefore, we suggest echocardiography should be performed in cases of clinical suspicion and family history rather than routinely in this patient group.